1. A. Transthoracic echocardiogram (ECHO) in patient with double outlet right ventricle with subaortic VSD. Left image: Subcostal four-chamber view with anterior angulation demonstrates both great arteries arising from the right ventricular outflow tract (RVOT) with aorta (A) to the right and pulmonary artery (P) to the left. Right image: Slight posterior angulation of the scan plane reveals that the ventricular septal defect (VSD) (arrow) is closely related to the aorta. There is infundibular muscle interposed between the VSD and the pulmonary the pulmonary valve. There is no pulmonary or subpulmonary stenosis and no important structures or valve attachments lying between the VSD and the aortic valve. Repair in this situation requires placement of an intraventricular tunnel to direct left ventricular outflow across the VSD toward the aorta and allowing the right ventricle to eject anteriorly to the patch directly into the pulmonary artery. B. Transthoracic echocardiogram illustrating double outlet right ventricle with remote VSD. Subcostal four chamber view with anterior angulation demonstrates both great arteries arising from the RVOT. Note, however, that the semilunar valves are relatively further away as compared to the ECHO shown in A. The pulmonary valve is to the left and is separated from the VSD by a thick wall of malposed infundibular septum (asterisk). The tricuspid valve has an outlet attachment that is positioned between the VSD and the aortic valve (arrow). Simple septation in this case is not possible. The three options for complete repair here would be to: (1) perform a complex intraventricular tunnel (multiple patch technique) and tricuspid valve replacement; (2) arterial switch operation with resection of the leftward portion of the malpositioned infundibular septum; and (3) treat this complex anatomical DORV variant as a functionally univentricular heart and channel the patient down a Fontan palliation strategy.
Source: Double Outlet Right Ventricle, Johns Hopkins Textbook of Cardiothoracic Surgery
Citation: Yuh DD, Vricella LA, Yang SC, Doty JR. Johns Hopkins Textbook of Cardiothoracic Surgery; 2014 Available at: Accessed: November 15, 2017
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